Optimal nutrition support has been integral in the management of cystic fibrosis (CF) since the disease was initially described. Nutritional status has a clear relationship with disease outcomes, and malnutrition in CF is typically a result of chronic negative energy balance secondary to malabsorption. As the mechanisms underlying the pathology of CF and its implications on nutrient absorption and energy expenditure have been elucidated, nutrition support has become increasingly sophisticated. Comprehensive nutrition monitoring and treatment guidelines from professional and advocacy organizations have unified the approach to nutrition optimization around the world. Newborn screening allows for early nutrition intervention and improvement in short- and long-term growth and other clinical outcomes. The nutrition support goal in CF care includes achieving optimal nutritional status to support growth and pubertal development in children, maintenance of optimal nutritional status in adult life, and optimizing fat soluble vitamin and essential fatty acid status. The mainstay of this approach is a high calorie, high-fat diet, exceeding age, and sex energy intake recommendations for healthy individuals. For patients with exocrine pancreatic insufficiency, enzyme replacement therapy is required to improve fat and calorie absorption. Enzyme dosing varies by age and dietary fat intake. Multiple potential impediments to absorption, including decreased motility, altered gut luminal bile salt and microbiota composition, and enteric inflammation must be considered. Fat soluble vitamin supplementation is required in patients with pancreatic insufficiency. In this report, nutrition support across the age and disease spectrum is discussed, with a focus on the relationships among nutritional status, growth, and disease outcomes.
In April 2020, a newly recognized pediatric disorder associated with COVID-19 characterized by significant inflammation with symptoms resembling Kawasaki disease was described by medical teams in the United States, the United Kingdom, and Italy. Before these reports, data from the initial COVID-19 outbreaks in China had not found the virus to cause significant morbidity or mortality in children. To date, pancreatitis has not yet been reported in either acute SARS-CoV-2 infection in children or the subsequent inflammatory syndrome. We describe a patient who presented with acute pancreatitis before rapidly progressing to multisystem organ dysfunction consistent with multisystem inflammatory syndrome in children due to COVID-19. Clinicians should be aware that in the context of the COVID-19 pandemic, pancreatitis can be an early presentation of multisystem inflammatory syndrome in children.
Eosinophilic esophagitis (EoE) is a chronic, immune-mediated disease that presents with symptoms of esophageal dysfunction, which vary by age. Diagnosis is made by upper endoscopy with esophageal biopsies to identify dense eosinophilic inflammation with at least 15 eosinophils per high-power field. Untreated, EoE can progress from inflammatory to esophageal remodeling with fibrosis and stricture formation. Food antigens are the primary trigger of inflammation in EoE. The most common food antigen triggers are dairy, wheat, egg, and soy. EoE can be managed with steroids or dietary elimination of food triggers. Elimination diets differ by the number of foods removed with specific nutrition implications for each diet. In addition, patients receiving swallowed steroids may have feeding dysfunction and need support for growth and nutrition intake. A multidisciplinary approach to care, including a dietitian, is integral to EoE management.
Background Intra-abdominal abscesses (IAA) are complications of Crohn’s disease, which often result in hospitalization, surgery, and increased cost. Initial management may include medical therapy, percutaneous drainage (PD), or surgery, although the optimal management of IAA in children is unclear. Methods Retrospective review of all pediatric patients with Crohn’s disease who developed an IAA from January 1, 2000 to April 30, 2012. Three groups, based on initial IAA treatment modality (medical, PD, and surgery), were compared. Results Thirty cases of IAA were identified (mean age at IAA diagnosis, 15.4 ± 2.6 yr, 67% female, median Crohn’s disease duration, 2.6 mo). Computed tomography was the most common initial (93%) and follow-up (47%) imaging. The average time to follow-up imaging was 8.5 days. For initial management, 18 received medical therapy, 10 PD, and 2 had surgery. The medical therapy group received more computed tomography scans for follow-up imaging than the PD group (12 [67%] versus 2 [20%], P = 0.046). There were no significant differences in abscess characteristics or management of posttreatment course between these 2 groups. Surgical resection occurred in 3 patients (17%) in the medical group and 2 (20%) in the PD group during index hospitalization. No significant differences were identified among treatment groups for readmissions, complications, or abscess recurrence. By 1 year, 12 of the 18 medically managed patients (67%) had surgery, and 6 of the 10 patients (60%) treated with initial PD ultimately had surgery. Conclusions The majority of patients with IAA require definitive surgical treatment, and there were no clear predictors of those who did not.
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